Periodic Fever with Pharyngitis, Aphthous Stomatitis and Cervical Adenitis Syndrome: A Rare Cause of Fever in Adults

Marques, Diogo Paixão; Rocha, Sofia; Manso, Marta Catarino; Domingos, Raquel

doi:10.12890/2019_001041

Cited by 5 publications

(7 citation statements)

References 5 publications

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“…The diagnosis of this disease is based on clinical findings and an age of the onset earlier than 5 years of age is considered an important diagnostic criterion, although there are studies describing a later onset of the symptoms. [20][21][22][23][24][25][26]…”

Section: Discussionmentioning

confidence: 99%

PFAPA Syndrome: Clinical, Laboratory and Therapeutic Features in a Single-Centre Cohort

Lazea¹,

Damian²,

Vulturar³

et al. 2022

IJGM

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The aim of this study is to describe a group of Romanian children with periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome. Materials: This consisted of 39 children diagnosed with PFAPA syndrome according to Thomas' criteria (eight patients with an age at diagnosis <1 year and 31 patients with an age at diagnosis >1 year). Methods: Retrospective analysis of the patients with PFAPA syndrome was focused on clinical features, laboratory findings and therapeutic methods. Comparison between the two groups divided by age at onset was also investigated. Results: Median age at onset was 1.58 years, and median age at diagnosis was 2.97 years. The mean interval between episodes was 35.5 days and the mean duration per febrile episode was 4.1 days. The median diagnosis delay was 2.42 years. The patients presented pharyngitis (100%), adenitis (94.8%) and aphthous lesions (66.7%). The frequency of febrile attacks was higher in children with an age at diagnosis under 1 year (p = 0.0287). Younger age was associated with the presence of aphthae. The mean value of C-reactive protein (CRP) was 7.9mg/dl and the mean value of leucocytes was 14,839/mm 3 . In 95% of patients given oral corticosteroids, remission of symptoms was reported within 24 h. In three patients, tonsillectomy was performed with complete remission of the disease. Conclusion:We present a cohort of children with PFAPA syndrome, with clinical and laboratory features similar to those described in the literature. Febrile attacks had a higher incidence in children with younger age at the onset of the disease. The patients had a favorable response to corticosteroids.

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Section: Discussionmentioning

confidence: 99%

PFAPA Syndrome: Clinical, Laboratory and Therapeutic Features in a Single-Centre Cohort

Lazea¹,

Damian²,

Vulturar³

et al. 2022

IJGM

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“…The first description of recurrence in adults with a previous remission of PFAPA during childhood was published in 2016, and it ushered in the notion that PFAPA might not be as self-limiting as once thought [ 19 ]. To our knowledge, only one other case of adult PFAPA has been reported in Portugal [ 20 ]. The patient was a 24-year-old female who had experienced pertinent symptoms in the previous four years prior to diagnosis.…”

Section: Discussionmentioning

confidence: 99%

Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome: A Rare Etiology of Fever in Adults

Cristina¹,

Costa

Toscano³

et al. 2021

Cureus

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We report the case of a 28-year-old male, with a past history of recurrent pharyngitis and tonsillectomy, who presented to the emergency department with fever, pharyngitis, and cervical adenitis. Inflammatory markers were elevated and the patient was initially started on ceftriaxone with remission after four days. However, the symptoms recurred three weeks later and an autoinflammatory disease was suspected. After exclusion of other illnesses, a diagnosis of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome was confirmed. The patient was successfully treated with a single dose of 60 mg of prednisolone at the beginning of the flare.PFAPA syndrome has been classically diagnosed solely in children but cases in adults are being increasingly recognized. Despite the increasing evidence of the delayed onset of PFAPA syndrome during adulthood, no specific tools are available to detect it and diagnosis is currently based on clinical diagnostic criteria, which have very low specificity and are tailored to pediatric patients. This case report stresses the need to consider this entity seriously despite its rarity, even among the adult population, so as to reduce iatrogenesis, start appropriate therapy in a prompt manner, and improve the quality of life of PFAPA patients.

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“…The exact cause of PFAPA syndrome is uncertain, though it is believed to be an autoinflammatory condition 2,4-6,13. It was originally thought that PFAPA was correlated with cryptic tonsillitis and that acute flares were actually the result of bacterial infection, but data did not indicate that acute symptoms were affected by antibiotic use; therefore, tonsillar flora or infection has been discounted as a cause of PFAPA 6.…”

Section: Pathophysiologymentioning

confidence: 99%

“…

eriodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is a disease of regularly recurrent fevers associated with other symptoms such as sore throat, shallow oral ulcers, and enlargement of the cervical lymph nodes. [1][2][3][4][5][6][7] The disease now known as PFAPA was originally referred to eponymously as Marshall syndrome based on its initial description in a 1987 article by Marshall and colleagues in The Journal of Pediatrics. The article described 12 pediatric patients with recurrent fever accompanied by headache, adenopathy, and oral ulcers.

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confidence: 99%

PFAPA syndrome in children

Gardner

2023

Jaapa

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Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is, as the name implies, characterized by an extremely regular cycle of fevers that is accompanied by one or more other symptoms such as oral ulcers, pharyngitis, adenitis, tonsillitis, sore throat, cervical adenopathy, and headache. Originally known as Marshall syndrome, PFAPA is most commonly identified in children younger than age 5 years; however, adults may also present with the disease, though they may report additional symptoms. PFAPA is now understood to be a diagnosis of exclusion. Laboratory studies are typically unremarkable except for increases in acute phase reactants such as C-reactive protein. Treatment is primarily supportive and most frequently uses systemic steroids to suppress the inflammatory response. Acute flares are self-limited, and the syndrome typically resolves on its own as the child reaches age 7 or 8 years.

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Periodic Fever with Pharyngitis, Aphthous Stomatitis and Cervical Adenitis Syndrome: A Rare Cause of Fever in Adults

Cited by 5 publications

References 5 publications

PFAPA Syndrome: Clinical, Laboratory and Therapeutic Features in a Single-Centre Cohort

PFAPA Syndrome: Clinical, Laboratory and Therapeutic Features in a Single-Centre Cohort

Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome: A Rare Etiology of Fever in Adults

PFAPA syndrome in children

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